Amyotrophic Lateral Sclerosis

1
Amyotrophic Lateral Sclerosis

• By David Likar

2
Background and History

• Also known as ALS, MND (Motor Neruon Disease), or
  Lou Gehrigs Disease.
• ALS/MND was first described in 1869 by
  Jean-Martin Charcot, a French neurologist.²
• Brought to national attention when baseball
  legend, Lou Gehrig, was diagnosed in 1939.

Lou Gehrig at bat.
Jean-Martin Charcot
3
What is ALS?

• ALS is a disease that attacks a persons motor
  neurons which eventually destroy the persons
  ability to use their muscles.
• It does not affect sight, smell, hearing, touch
  or taste.¹
• Because it only affects motor neurons, it does
  not affect intelligence, memory, or personality.¹

4
What Happens To A Person With ALS?

• ALS slowly destroys the ability for the brain to
  control voluntary movement. They lose the
  strength to move their arms, legs, and body.
• Eventually, The diaphragm and chest wall are
  affected and breathing becomes impossible without
  a respirator. This happens within 3-5 years.¹
• 20 of people with ALS will leave up to 5 years
  and 10 will live up to ten years.¹

5
Symptoms?

• Early symptoms occur where motor neurons are
  damaged first.
• Some people have damage in one leg and start to
  stumble more often and feel awkward while the
  walk or run.²
• Some have trouble with their hands and begin
  having trouble writing, buttoning their shirt or
  turning a key in a lock.¹
• Some notice speech problems.

6
Causes?

• The causes of ALS are currently unknown. 90-95
  of cases occur randomly.
• Only 5-10 of cases show some signs of heredity.³
• Genes associated to ALS have been identified.³
• One theory is that people with ALS, have
  overactive immune systems which attack their
  motor neurons as if they were viruses.¹

7
Diagnosis?

• Diagnosis of ALS is very difficult because ALS
  mimics many other neurological diseases and
  disorders such as, multiple sclerosis, spinal
  cord tumors or nerve compression.¹
• ALS is usually diagnosis by ruling out other
  possible diseases by use of EMGs, NCVs, MRIs, and
  CAT scans.¹

8
Prevalence?

• 20,000 people are currently diagnosis with ALS,
  and another 5,000 are diagnosis each year in the
  US.
• ALS affects people of all races and ethnic
  backgrounds.
• It is more prevalent in men than women.
• It usually affects people from ages 40-60 years
  old.

9
Treatment?

• There is currently no cure for ALS.
• A drug called Riluzole is used to prevent motor
  neuron damage and prolongs a patients life by
  several months.
• A series of professionals such as doctors,
  nurses, speech pathologists, therapists, and
  social workers are used to provide treatment.

4
4
10
Citations

• Medline Plus, National Institute of Health,
  http//medlineplus.gov/
• ALS Association website, http//www.alsa.org/
• Leavitt, B.R. (2002). Hereditary motor neuron
  disease caused by mutations in the ALS2 gene
  The long and the short of it. Clinical
  Genetics, 62 (4), 265-269
• Ginsberg, G. Lowe, S. (2002). Cost
  effectiveness of treatments for amyotrophic
  lateral sclerosis a review of the literature.
  Pharmacoeconomics, 20 (6), 367-388